study of ventilatory mechanics in patients with sickle cell anemia

Conditions: Sickle Cell Anemia

Summary: the sickle cell anemia (sca) is a recessive genetic condition, monogenic, resulting in defects in the red cell structure. in the investigators' country, this disease affects about 3,000 children each year and is considered one of the most prevalent disorders among the group of existing hereditary diseases. the lungs are frequently affected in this disease by acute chest syndrome (sta). besides being the leading cause of death and the second leading cause of hospitalization in sca, the sta is correlated with cognitive impairment frame these patients, resulting secondary stroke vaso-occlusion of capillaries that supply the brain tissue. traditional tests of pulmonary function allow assess whether the person has any commitment in the respiratory system, whether obstructive, restrictive or mixed. to run these tests it is necessary that the patient understands and performs a forced expiratory maneuver to obtain reliable results. in the particular case of sca, performing these tests it is very difficult due to the presence of cognitive impairment of varying degrees. this results in underdiagnosis of early changes in the lung parenchyma during the therapeutic window, committing the proper monitoring and treatment offered to these patients.

the aim of the present study is to observe the use of the forced oscillation technique (fot) in the detection of the alterations of the respiratory mechanical in subjects with sickle cell anemia. one group of patients with sickle cell anemia was compared with a volunteers group without pulmonary alterations. the experimental phase of this research was developed from the university hospital of the pulmonology department pedro ernesto (hupe) and laboratory instrumentation biomedical of rio de janeiro state university (uerj) and was approved by the committee for ethics in the hospital survey. the informed consent was obtained from all volunteers and patients. the study protocol complies with the helsinki declaration and resolution cns 466/12. patients were recruited from the hematology clinic of the university hospital pedro ernesto. after screening of the inclusion criteria, these individuals were referred to the following tests: respiratory function support and technical by forced oscillations. the respiratory function test were performed in the pulmonary function testing laboratory at the university hospital pedro ernesto and obeyed the standard protocol of the american thoracic society / european respiratory society (ats / ers). in clinical exams with the fot, the volunteers remained seated, his head in the neutral position and used a nose clip. they were asked to maintain a basal respiration throughout the examination period through a silicone nozzle, with cheeks sustained by the hands in order to reduce the shunt effect. the system applies sinusoidal pressure signals emitting oscillatory waves in the frequency range 4-32 hz. the results to be considered suitable, should apre sit coherence function ≥ 0.9. three measurements were performed for 16 seconds, with rest interval between them. finally, to analyze the parameters measured, obtained was the average of all the results. among the parameters evaluated the resistive and reactive parameters of the respiratory system were studied, respectively.

Eligibility criteria:
inclusion criteria: * patients older than 18 years * typing of hemoglobin ss exclusion criteria: * history absence of respiratory infections and hospitalized in the last three months * asthma , smoking, rheumatological and oncological diseases, acute pain and inability to independent ambulation